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Mouse Anti-TIMM8A Recombinant Antibody (9G347) (CBMAB-D2276-YC)


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Summary

Host Animal
Mouse
Specificity
Human
Clone
9G347
Antibody Isotype
IgG
Application
ELISA, IHC, WB

Basic Information

Immunogen
Partial recombinant protein of human deafness dystonia protein 1 (a.a.9-98) with a GST tag
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
translocase of inner mitochondrial membrane 8 homolog A (yeast)
Introduction
TIMM8A is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. TIMM8A is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in TIMM8A also cause Jensen syndrome; an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. TIMM8A, along with TIMM13, forms a 70 kDa heterohexamer.
Entrez Gene ID
1678
UniProt ID
O60220
Alternative Names
Translocase Of Inner Mitochondrial Membrane 8A; X-Linked Deafness Dystonia Protein; Deafness Dystonia Protein 1; DDP1; DDP; Translocase Of Inner Mitochondrial Membrane 8 (Yeast) Homolog A; Translocase Of Inner Mitochondrial Membrane 8 Homolog A (Yeast); Mitochondrial Import Inner Membrane Translocase Subunit Tim8 A;
Function
Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins. Probably necessary for normal neurologic development.
Biological Process
Biological Process nervous system developmentSource:ProtInc1 Publication
Biological Process protein insertion into mitochondrial inner membraneSource:BHF-UCL1 Publication
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Mohr-Tranebjaerg syndrome (MTS):
An X-linked recessive disorder characterized by postlingual sensorineural deafness with onset in early childhood, dystonia, spasticity, dysphagia, mental deterioration, paranoia and cortical blindness.
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For research use only. Not intended for any clinical use.

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